Journal article
Thoracic Sertoli–Leydig cell tumor: An alternative type of pleuropulmonary blastoma associated with DICER1 variation
Pediatric blood & cancer, Vol.68(11), pp.e29284-n/a
11/2021
DOI: 10.1002/pbc.29284
PMCID: PMC8463509
PMID: 34398502
Abstract
A 2‐year‐old boy presented with a large cystic and solid chest mass arising from the lung, radiographically consistent with pleuropulmonary blastoma (PPB). He underwent right lower lobectomy with resection of a well‐circumscribed, mixed solid and cystic mass. The solid areas were composed of cords and nests of tumor cells in the myxoid stroma and retiform foci whose pathologic and immunophenotypic findings were consistent with a sex cord‐stromal tumor with features of a Sertoli–Leydig cell tumor. Tumor testing showed a pathogenic variant in the DICER1 RNase IIIb hotspot domain. Family history was suggestive of DICER1 germline pathogenic DICER1 variation in absence of a detectable germline variant. He received 12 cycles of chemotherapy with ifosfamide, vincristine, dactinomycin and doxorubicin (IVADo) and surgery with complete response. One year after completion of chemotherapy, imaging studies showed concern for recurrence confirmed by thorascopic biopsy of a pleural‐based mass. He is currently receiving cisplatin‐based chemotherapy with reduction in tumor size. Review of the literature showed no similar cases; however, review of our pathology files revealed a single similar case of anterior mediastinal Sertoli cell tumor in a 3‐year‐old girl.
Details
- Title: Subtitle
- Thoracic Sertoli–Leydig cell tumor: An alternative type of pleuropulmonary blastoma associated with DICER1 variation
- Creators
- William Terry - Division of Pediatric Hematology/Oncology, Stead Family Children's Hospital, Iowa City, Iowa, USA.Erica M. Carlisle - Division of Pediatric Surgery Stead Family Children's Hospital Iowa City Iowa USAPaige Mallinger - International PPB/DICER1 Registry Children's Minnesota Minneapolis Minnesota USAAlexander T. Nelson - International PPB/DICER1 Registry Children's Minnesota Minneapolis Minnesota USADavid Gordon - Division of Pediatric Hematology/Oncology, Stead Family Children's Hospital, Iowa City, Iowa, USA.Yoav H. Messinger - International PPB/DICER1 Registry Children's Minnesota Minneapolis Minnesota USAAmanda Field - ResourcePath, Arlington, Virginia, USA.Louis P. Dehner - Washington University Medical CenterD. Ashley Hill - Children's NationalKris Ann P. Schultz - International PPB/DICER1 Registry Children's Minnesota Minneapolis Minnesota USA
- Resource Type
- Journal article
- Publication Details
- Pediatric blood & cancer, Vol.68(11), pp.e29284-n/a
- DOI
- 10.1002/pbc.29284
- PMID
- 34398502
- PMCID
- PMC8463509
- NLM abbreviation
- Pediatr Blood Cancer
- ISSN
- 1545-5009
- eISSN
- 1545-5017
- Number of pages
- 5
- Language
- English
- Date published
- 11/2021
- Academic Unit
- Stead Family Department of Pediatrics; Surgery; Hematology/Oncology
- Record Identifier
- 9984322936902771
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